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2020 Posterior Reversible Encephalopathy Syndrome (PRES) Presentation Diagnosis and Treatment
2020 Posterior Reversible Encephalopathy Syndrome (PRES) Presentation Diagnosis and Treatment
2020 Posterior Reversible Encephalopathy Syndrome (PRES) Presentation Diagnosis and Treatment
Medicine, Guru Teg Bahadur ABSTRACT PRES can be considered to be the basis of the
Hospital, New Delhi, India Posterior reversible encephalopathy syndrome (PRES) is neurological manifestations of preeclampsia/
a neurological disorder which is characterised by variable eclampsia.8 Cases can present in very early preg-
Correspondence to
Dr Anant Parasher, Medicine,
symptoms, which include visual disturbances, headache, nancy (before the 20th week of gestation), as well
Guru Teg Bahadur Hospital, vomiting, seizures and altered consciousness. The exact as rarely in the late stages of pregnancy with intrau-
New Delhi 110095, India; pathophysiology of PRES has not been completely terine death.9 Severe pre-eclampsia (defined as
Breakdown of the blood brain barrier and endothelial dysfunc- hemianopsia and cortical blindness, to altered consciousness
tion occurs in PRES with fluid and macromolecule extravasation presenting as mild confusion, agitation or coma. Other symp-
into the interstitium. Increased concentrations of circulating cyto- toms may include nausea, vomiting and seizures. Status epilep-
kines (eg, tumour necrosis factor α, interleukin 1 and endothelin 1) ticus is common, which may be generalised. Non-convulsive
activate endothelial cells and allow interaction and adhesion of status can be prolonged and last for days in PRES and should
circulating leucocytes(figure 2). The tight junctions are disrupted be carefully observed. Drug intoxication and psychosis should
and vascular endothelial growth factor expression is increased, be ruled out in these cases, so that treatment can initiated as
leading to increased vascular permeability and vasogenic oedema. early as possible.5
To complicate the matter further, not all the patients with The most common symptoms seen in obstetric patients are
PRES have hypertension, and cytotoxicity is thought to be seizures (45%), visual disturbances (34%), alteration of con-
the mechanism underlying cerebral oedema in these patients. sciousness (19%)1 and focal deficits (4%).21 The degree of hyper-
The associated conditions include cytotoxic therapies (eg, tension is not associated with the extent of cerebral lesions and
ciclosporin, tacrolimus), infection/sepsis/shock, autoimmune oedema can also occur at lower levels of arterial blood pressure.
disease and exposure to toxic agents.6 18 19 The mechanism This is chiefly due to ongoing endothelium damage, as indicated
might be direct toxicity to vascular endothelium leading to by the high lactic acid dehydrogenase (LDH) levels in laboratory
capillary leakage and breakdown of the blood brain barrier, tests.22 23
which triggers vasogenic oedema.2 The damage may also be
seen with non-toxic levels of these drugs. Imaging studies
Severe anaemia can be a predisposing factor for PRES due to the The most common location of the lesions in PRES is the parietal-
endothelial dysfunction caused by insufficient oxygen supply. This occipital lobe or ‘posterior’ area of the brain. Lesions may also be
can further damage and disrupt the blood brain barrier.20 Rapid observed in the anterior regions, basal ganglia, brainstem and the
blood transfusion in these patients may cause a rapid increase in cerebellum.1 24 25 The characteristic imaging patterns in PRES are
total blood volume, with resultant cerebral blood flow overload. represented in box 2.26 Symmetrical white matter abnormalities
This acute cerebral hyperperfusion disrupts cerebral autoregulation suggestive of oedema may be seen in the CT and MRI scans, but
and might result in the vasogenic oedema found in PRES.5 not exclusively in the posterior parieto-occipital regions of the
cerebral hemispheres.1 27 28
Clinical presentation Diffusion-weighted imaging is essential to distinguish
The symptoms of PRES are variable, ranging from visual dis- between vasogenic and cytotoxic oedema.1 29 Diffusion-
turbances which may present as blurred vision, homonymous weighted MRI is the modality of choice for confirming the
diagnosis of PRES(figure 3) and to differentiate between
reversible vasogenic and irreversible cytotoxic oedema, as
compared with a CT scan, which can be normal in some
cases of PRES. Radiologically detectable cerebral lesions may
persist in some cases in spite of intensive monitoring and
prompt aggressive therapy.1
► Holo-hemispheric watershed.
► Superior frontal sulcus.
Figure 3 MRI with T2-flair-weighted images showing the typically
► Dominant parietal/occipital.
hyperintense bilateral lesions indicating vasogenic oedema in the par-
► Partial and/or asymmetric PRES.
ieto-occipital regions as well as less common lesions in the frontal
regions and brain stem (arrows).27 28 PRES, posterior reversible encephalopathy syndrome.